Abstract
Over the last two decades molecular studies of inherited tumor syndromes that are associated with the development of kidney cancer have led to the identification of genes and biochemical pathways, which play key roles in the malignant transformation of renal epithelial cells. Some of these findings have broad biological impact and extend beyond renal cancer. This review's focus is on the von Hippel-Lindau (VHL)/hypoxia-inducible factor (HIF) oxygen-sensing pathway and its role in physiology, energy metabolism and tumorigenesis.